Endo Hiroyasu, Rees Terry D, Niwa Hideo, Kuyama Kayo, Oshima Maya, Serizawa Tae, Tanaka Shigeo, Komiya Masamichi, Ito Takanori
Department of Oral Diagnosis Nihon University School of Dentistry at Matsudo Matsudo Japan.
Department of Periodontics Texas A&M College of Dentistry Dallas Texas USA.
Clin Exp Dent Res. 2019 Mar 4;5(3):219-224. doi: 10.1002/cre2.174. eCollection 2019 Jun.
Pemphigus vulgaris (PV) is an autoimmune, blistering disease that affects the mucosa and skin. The current theory favors the concept that anti-desmoglein (Dsg) 3 autoimmunity is the only pathogenic event needed to induce acantholysis. However, a few cases of active PV in the oral cavity had no detectable anti-Dsg 3 antibody. The aim of this study was to evaluate the differences in clinical and laboratory findings, whether or not the anti-Dsg 3 antibodies were present. This study was based on a retrospective review of 10 PV cases. The evaluation of the circulating autoantibody titers to Dsg 3 was conducted by using enzyme-linked immunosorbent assay (ELISA). An index value of 20 or more was used as the cutoff for a positive reaction. Only five of the 10 PV cases had a positive Dsg 3 ELISA. There were no differences in clinical, cytological, histopathological, and direct immunofluorescence findings, whether or not the anti-Dsg 3 antibodies were present. Of the five patients with a negative reaction at the time of diagnosis, the Dsg 3 ELISA became positive in the follow-up period in three cases. In the remaining two cases, the Dsg 3 ELISA was consistently negative for 18 months. Dsg 3 ELISA was negative early in some PV cases. Therefore, PV acantholysis may precede the elevation of circulating anti-Dsg 3 antibody levels. The diagnosis of PV should be considered based on comprehensive clinical, histopathological, and immunofluorescent criteria.
寻常型天疱疮（PV）是一种影响黏膜和皮肤的自身免疫性水疱病。目前的理论支持这样一种观点，即抗桥粒芯糖蛋白（Dsg）3自身免疫是诱导棘层松解所需的唯一致病事件。然而，少数口腔活动性PV病例未检测到抗Dsg 3抗体。本研究的目的是评估抗Dsg 3抗体是否存在时临床和实验室检查结果的差异。本研究基于对10例PV病例的回顾性分析。采用酶联免疫吸附测定（ELISA）检测循环中抗Dsg 3自身抗体滴度。以20或更高的指数值作为阳性反应的临界值。10例PV病例中只有5例Dsg 3 ELISA呈阳性。抗Dsg 3抗体是否存在，临床、细胞学、组织病理学和直接免疫荧光检查结果均无差异。诊断时反应阴性的5例患者中，3例在随访期间Dsg 3 ELISA转为阳性。其余2例中，Dsg 3 ELISA在18个月内一直为阴性。在一些PV病例中，早期Dsg 3 ELISA为阴性。因此，PV棘层松解可能先于循环抗Dsg 3抗体水平升高。PV的诊断应基于综合的临床、组织病理学和免疫荧光标准。
